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What antibodies are included in the Rheumatoid Factor (RF)?
IgM, IgG and rarely IgA
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The titer of RF in a patient suggests:
The probability that a patient has RA as well as the severity of the disease. However, RA can occur without RF.
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What antibodies are included in Anti-cyclic citrulline antibodies?
IgM and IgG, which bind to keratin. AKA filaggrin. This is more specific for RA than RF.
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What are the specific agents that contribute to local inflammation? (2)
- Tumor necrosis factor alpha and Interleukin 1 (IL-1). These agents:
- activate matrix proteases
- stimulate PGE2 production
- activate osteoclasts (happens long term)
- Contribute to systemic feature of RA
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What is the hallmark of RA?
- Proliferative synovial pannus: a destructive inflammatory VASCULAR granulation tissue that extneds form the synovium to involve the bare area (region of unprotected bone)
- Note: an MRI can distinguish between plannus and cartilage.
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A subchondral cyst formation occurs as a result of inflammation in what disease?
RA
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What is characterized by a mothy appearance on xray (less xray dense) in RA?
Juxtaarticular osteopenia
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What is the difference in joint space on an xray between RA and OA?
- RA: joint space widening
- OA: joint space narrowing
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What needs to met in the diagnostic criteria to diagnose RA?
The last 4 of the 7 criteria for RA need to be met to meet the diagnostic criteria of RA for the American Rheumatism Association.
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How many joints/joint areas must be involved in RA?
3 joint areas
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What specific joints in the hands are affected by RA?
PIP, MCP (DIP joints usually spared)
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What type of deviation in the MCP joints is common in RA? in the wrist?
Ulnar deviation, radial deviation respectively.
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What is characterised by PIP hyperextension and DIP flexion?
- Swan neck deformity.
- Note: boutonniere deformity is PIP flexion and DIP extension
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What is specific to the the joint disease in the knee and surrounding tissue in RA? (2)
See atrophy of periarticular muscles (quads) and possibly a baker's cyst
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Which spinal vertebrae are most affected in RA?
Cervical
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What is the most common extra-articular manifestation of RA?
Rheumatoid nodules: granulomatous lesions that deveop around small blood vessels.
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What is the hallmark of early RA?
Vasculitis
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What pharmacologic treatment (3) are used and when for RA?
NSAIDS first, DMARDs next (methotrexate usually first), steroids for flares. TNF inhibitors are usually used in combination with DMARDs.
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Which gene is most associated with RA?
HLA-DR4
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What causes the advancement of RA?
angiogenesis
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What is the best marker that correlates with the ACTIVITY of the disease in RA?
ESR
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What type of disease is SLE?
Inflammatory AND autoimmune
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What race has the highest prevelence of SLE?
African Americans
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What is the pathologic gene in SLE?
Anti-DS DNA Ab
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What body system is almost always associated with SLE?
Renal
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What type of rash is associate with SLE?
Malar or butterfly
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Which joints are most commonly affected with SLE?
Knees (small joints of hands and knees are common).
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What is the tendon subluxation associated with SLE called?
Jacoud's classic
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SLE is associated with what type of vascular condition?
Hypercoagulable state: ACLA, elevated PTT, increase miscarriages
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What are the abnormal antibody tests? (4) and what specific test confirms diagnosis?
- ANA
- Anti smith (if this is positive it confirms diagnosis)
- DS DNA (strong association with kidney disease)
- Antiphospholipids
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What are the usual labs to order for SLE? (3)
- CBC, UA (look for any cell line decrease)
- BMP for BUN/Cr
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How many diagnostic criteria is required for diagnosis of SLE? (out of 11)
4/11
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What is the mainstay of treatment for SLE?
Oral steroids (prednisone)
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What is the first and most of symtoms in polymyositis (PM), also has in common with PMR?
Proximal muscle weakness
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Dermatomyostitis(DM) have an increased risk of what?
Cancer
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What type of skin manifestations are usually involved in PM and DM? (6)
Heliotrope rash, facial erythema, malar rash, shawl sign, gottron's papules (over PIP and MCP), possible raynaud phenomenon.
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What genes are positive for PM and DM?
- ANA
- Anti-jo-1 (if so think interstitial lung disease, especially with arthritis and fever)
- (also note that muscle enzymes are high- CK and aldolase)
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What is the treatment for PM and DM?
High dose steroids, methotrexate, azathioprine
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What are the 2 key diagnostic findings in sarcoidosis?
Bilateral hilar infiltrates and non caeseating granulomas
-
What type of problem in sarcoidosis? inflammatory or autoimmune?
more of an inflammatory problem
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What organ is most commonly associated with sarcoidosis?
Lung
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What is Lofgrens syndrome associated with and what does it entail?
sarcoidosis. includes: arthritis, erythema nodosum(painful red nodular rash on knees, which is also associated with Behcets dx), hilar adenopathy
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If sarcoidosis is in fact affecting the lung what specific level int he blood what you want to get?
ACE level. Need biopsy to be sure of diagnosis.
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How do you treat sarcodosis?
Oral Prednisone, high enough to control sxs.
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What are the 2 most common presenting symptoms of sjogrens disease?
Dry eye, dry mouth
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People Sjogrens disease are at increased risk for what?
lymphoma. also associated with vasculitis.
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What is the test for sjogren's disease used on the eye?
Schirmer's test
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What genes are associated with Sjogrens?
- Anti SSA
- Anti SSB
- HLA-DR, HLA-DQ
- Can often see: +ANA (most common test done), +RF, elevated ESR
-
What is first line treatment in autoimmune disease that is chronic and progressive?
DMARDs (usually cytotoxic or TNF-alpha blocking meds)
-
What the 2 most common drugs that cause SLE?
Hydralazine and procainamide
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What is the age group most commonly affected by JRA?
1-6yo
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What type of onset is the most common in JRA? (systemic, polyarticular or pauciarticular)
Pauciarticular (<5 joints involved)
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If a child is having a systemic onset of JRA what symtoms are likely seen? (6)
Spiking high fever (>102), salmon pike macular rash-koebners phenomenon (develop a rash where trauma), arthraglias/myalgias, hepatosplenomegaly, leukocytosis, Polyserositis
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In a polyarticular pattern onset of JRA, what part of the face is classically involved?
mandible- inflammation causes premature closure of growth plate and shortened jaw.
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What specific systemic feature in females is associated with pauciarticular arthritis?
Iridocyclitis: insidious and asymptomatic, can go blind if not treated. Make sure to get an opthalmology exam!! (slit lamp every 6mo)
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What is the first line treatment for JRA if relatively mild disease?
liquid NSAIDs. Can use DMARDs, but be careful with these because kids are rapidly growing.
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What are the main symptoms of Dermatomysitis?
Heliotrope rash, proximal muscle weakness (which is also seen in PM!!)
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What size vessels are described in Kawaski disease?
small to medium vessels (it is a vasculitis)
-
What is the clinical presentation of Kawasaki disease?
High fever, enlarged cervical lymph nodes, skin rash (polymorphous exanthem), arthralgia, strawberry tongue, S3, S4, pericarditis. Diagnosis is clinical remember.
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How do you treat Kawasaki disease?
Start before you are convinced of diagnosis, IVIG, Aspirin in high doses. Severe cardiac complications can arise if you do not initiate fat enough.
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What is the most common effected system in Henoch-Schonlein Purpura?
renal: glomerulonephritis
-
What causes HSP?
IgA deposition
-
What are clinical manifestations of HSP?
Palpable purpura, Abdominal pain, Renal disease, sometimes lung involvement, arthralgias/arthritis
-
How do you treat HSP?
Supportive! if symptomatic can give NSAIDs, acetaminophen. better in 1-6 weeks
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What does CREST stand for?
- C: calcinosis
- R: raynauds
- E: esophageal dysmotility
- S: sclerodactyly
- T: telangectasia
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If there is renal involvement with sceleroderma, what pharmacologic treatment should be initiated?
ACEI
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What the RED FLAGS that suggest vasculitis? (3)
Palpable purpura, hemotysis, glomerulonephritis
-
What is the prefered mode of treatment in vasculitis?
immune suppression
-
What is PMR linked to?
Giant cell arteritis
-
Decreased proximal strength is characteristic of...
GCA (giant cell arteritis)
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How do you treat Giant cell arteritis and PMR?
Prednisone
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What type of vasculitis is Takayasu's arteritis?
Large vessel, primarily aorta and branches of aorta
-
What is the treatment of Takayasu's arteritis?
Glucocorticoids or cytotoxic drugs like methotrexate
-
What 2 diseases are positive for ANCA (anti-neutrophil cytoplasmic autoantibodies)?
Wegeners and Churg-Strauss. Steroid treatment for both.
-
Which vasculitis is associated with Hep B and C?
Polyarteritis nodosa
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What is the hallmark for small vessel vasculitis?
Palpable purpura
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When there is no apparent cause for thromobic events or miscarriages, what should you suspect?
Anti-phospholipid syndrome
-
What type of disease is scleroderma?
fibroblast disease...NOT inflammatory.
-
How do you treat scleroderma?
early methotrexate, cyclophsphamide for ealy interstial lung disease, prostacyclin analogs for pulmonary HTN, vasodilators for raynauds, PPI for gerd, WILL NOT RESPOND TO STEROIDS!
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What gene is commonly found in Spondyloarthopathies?
HLA-B27...most commonly associated with AS
-
What is the hallmark of spondyloarthopathies?
Sacroiliitis
-
What joints are first involved in AS?
SI joints
-
When imaging the back for AS what is characteristic to be found on xray?
Bamboo spine
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What does AS look exactly like on xray, but have nothing else in common with?
DISH (diffuse idopathic skeletal hyperostosis)
-
What is commonly associated with spondyloarthropathies?
uveitis
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What is the triad of eye inflammation of Reiter's disease?
- Uveitis, urethritis, oligoarthritis
- (Reactive arthritis is an incomplete triad, but contains olgoarthitis)
-
What is the clinical presentation of Reiters/reactive arthritis?
After STD or gastroeneritis, culures are negative, olioarthritis, enthesopathy, ulcers (painful)
-
What is seen the hands and feet of PSA?
Dactilitis and nail changes (pitting and ridging)
-
Which joints are most commonly involved with PSA?
DIPs, spine and sacroiliac joints
-
Which spondyloarthropathy is this? primarily back symptoms, and losing motion in back?
AS
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What is the most common bug causing nongonococcal arthritis?
S. aureus
-
Which joint does nongonococcal arthritis most commonly effect?
Knee
-
How do you treat nongonococcal arthritis?
Antibiotics (4-8 weeks and start with oxacillin or 3rd gen ceph), drain joint (if in hip use arthrotomy otherwise use arthrocentesis), rest, ASAP active ROM to preserve joint
-
If acute monoartivular arthritis and <35 yo it is____until proven otherwise!
GC
-
What is the sequence of symptoms in gonococcal arthritis?
First migrating polyarthritis then either tenosunovitis or purulent monoarthritis (knee most common)
-
What is gold standard to diagnose gonococcal arthritis?
Culture it!
-
How do you treat gonocococcal arthritis?
admit, 48 hour of IV cephtriaxone then cephalosporins for 7-10 days...Dont forget to treat for chlaydia too!!
-
What disease is often associated with fibromyalgia?
IBS
-
What is the most common rhuematologic illness? second?
OA followed by fibromyalgia
-
What is the primary symptom of fibromyalgia....secondary?
primary symptom is pain, secondary symptom is fatigue
-
In terms of the heart, what condition is associated with fibromyalgia?
MVP
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What other tests should you be sure to check before diagnosing fibromyalgia? (7)
Anemia, TSH, CPK, ESR, ANA, SLE, RF
-
How many of the trigger points must be felt for fibromyalgia? /18
11/18
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What is the main differences between CFS and fibromyalgia?
- CFS: fatigue and usually gets better
- Fibromyalgia: Pain, and usually does not get better
-
What is the difference between acute inflammation and chronic inflammation?
- Acute is a vascular response with vasodilation
- Chronic releases cytokines, causing scar formation
-
What rheumatologic illnesses are more common in men?
Gout, spondyloarthopathy, nongonococcal arthritis
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What level of WBCs is normal in a joint?
<200. >50,000 is assumed to be infected.
-
What is a type I hypersensitivity reaction?
immediate hypersensitivity disorders
-
What is a type II Hypersensitivity disorder?
antibody-mediated disorder
-
What is a type III hypersensitivity disorder?
immune complex mediated disorder (what RA is)
-
What is a type IV hypersensitivity disorder?
T-cell mediated disorder (like PPD)
-
Uric acid is the end product of____
Purine
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What are the 4 ways that gout can result from?
- 1. overproduction of purines
- 2. decrease salvage of free purine bases
- 3. changin breakdown of nucleic acids and increase cell turnover
- 4. decrease urinary excretion (primary and most common 90%)
-
What type of areas does gout tend to precipitate in?
cooler areas of the body, like the big toe.
-
What type of formation is eventually formed after 10 years of having the first gout attack?
Tophi formation (called chronic tochaceous gout at that point)
-
What is typical treatment for gout?
- Acute attack: Colchicine, NSAIDS, steroids
- Rx the uric acid: Allopurinol, probenecid.
- Indomethacin in a young person works great! NSAIDS but not chronically. Colchicine can be used for acute attacks if used early and can also be used prophylactically, high dose aspirin for pain, steroids, ACTH
-
What is the gold standard for diagnosing gout?
Synovial fluid analysis to see cystals in the fluid.
-
At what level of uric acid in your body will you start crystalizing?
6.8
-
What is uric acid levels also a marker for? (what syndrome)
Metabolic syndrome
-
If someone is a profound overproducer of uric acid, what med do you NEVER want to give them?
Probenecid
-
What is psuedogout commonly associated with? (in severe disease)
OA. Cartilage degeneration occurs with shedding pyrophosphate crytals into joint cavity (old age)
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